PDF(Pubmed)
Abstract：
Concurrent malignancy and IgA nephropathy are rare. Despite the lack of solid experimental evidence, there are theoretical hypotheses of pathophysiology for the development of glomerular damage in cancer patients, like aberrant immune activities. Here, we describe a nine-year-old child who was admitted due to nephrotic syndrome. Abdominal imaging examination accidentally revealed a retroperitoneal tumor, and surgical resection was performed with a pathological diagnosis of neuroblastoma. However, complete removal of the tumor had no impact on the clinical manifestation of nephrotic syndrome, like proteinuria. The use of corticosteroids alone only led to a partial resolution of proteinuria, and resistance developed after one month of treatment. A further kidney biopsy was performed, which suggested IgA nephropathy. Clinical remission of IgA nephropathy was achieved after standard combination treatment of corticosteroids and mycophenolate mofetil for 10 months. This study represented the first case report of neuroblastoma associated with IgA nephropathy. We postulated that IgA nephropathy pathogenesis might be associated with neuroblastoma, though a coincidence of these two conditions cannot be fully excluded. Standard treatment for IgA nephropathy is applicable for patients with concomitant cancer.
摘要：
并发恶性肿瘤和IgA肾病很少见。尽管缺乏可靠的实验证据,对于癌症患者肾小球损伤的发展，有病理生理学的理论假设，比如异常的免疫活动。这里,我们描述了一名9岁儿童因肾病综合征入院。腹部影像学检查意外发现腹膜后肿瘤,手术切除后病理诊断为神经母细胞瘤。然而,肿瘤完全切除对肾病综合征的临床表现无影响,比如蛋白尿.单独使用皮质类固醇仅导致蛋白尿的部分消退，治疗一个月后出现耐药性。进行了进一步的肾脏活检，提示IgA肾病.在糖皮质激素和霉酚酸酯标准联合治疗10个月后，IgA肾病的临床缓解。这项研究代表了与IgA肾病相关的神经母细胞瘤的首例报告。我们推测IgA肾病的发病机制可能与神经母细胞瘤有关。尽管不能完全排除这两个条件的巧合。IgA肾病的标准治疗适用于伴随癌症的患者。
